Preferred Label : Idiopathic non-specific interstitial pneumonia;
ICD-11 definition : Idiopathic nonspecific interstitial pneumonia is one type of idiopathic interstitial
pneumonias (IIPs). Idiopthic nonspecific interstitial pneumonia has been associated
with many medical conditions, although a causal link has not been identified. In addition,
it may be idiopathic. Nonspecific Interstitial Pneumonia(NSIP) originated as a histopathologic
categorization reserved for surgical lung biopsies not demonstrating a clearly identifiable
pattern. The histopathologic pattern of NSIP is found in a wide variety of diseases
of known cause (e.g., hypersensitivity pneumonitis, drug-related, acquired immunodeficiency
syndrome [AIDS]–related, and collagen-vascular diseases). Moreover, many patients
diagnosed with idiopathic NSIP meet the case definition of undifferentiated connective
tissue disease, suggesting that idiopathic NSIP might actually be an autoimmune disease.
In addition, it is estimated that up to 15% to 20% of patients who present with a
chronic ILD either have an occult connective tissue disease or subsequently develop
a clinically overt connective tissue disease. In this particular group of patients,
the initial clinical presentation may be essentially indistinguishable from that of
several IIPs (especially NSIP and UIP).;
ICD-11 synonym : Nonspecific Interstitial Pneumonia; NSIP - [Nonspecific Interstitial Pneumonia];
ICD-11 acronym : NSIP;
Origin ID : 1319771917;
Automatic exact mappings (from CISMeF team)
Idiopathic nonspecific interstitial pneumonia is one type of idiopathic interstitial
pneumonias (IIPs). Idiopthic nonspecific interstitial pneumonia has been associated
with many medical conditions, although a causal link has not been identified. In addition,
it may be idiopathic. Nonspecific Interstitial Pneumonia(NSIP) originated as a histopathologic
categorization reserved for surgical lung biopsies not demonstrating a clearly identifiable
pattern. The histopathologic pattern of NSIP is found in a wide variety of diseases
of known cause (e.g., hypersensitivity pneumonitis, drug-related, acquired immunodeficiency
syndrome [AIDS]–related, and collagen-vascular diseases). Moreover, many patients
diagnosed with idiopathic NSIP meet the case definition of undifferentiated connective
tissue disease, suggesting that idiopathic NSIP might actually be an autoimmune disease.
In addition, it is estimated that up to 15% to 20% of patients who present with a
chronic ILD either have an occult connective tissue disease or subsequently develop
a clinically overt connective tissue disease. In this particular group of patients,
the initial clinical presentation may be essentially indistinguishable from that of
several IIPs (especially NSIP and UIP).
